Probable variant Creutzfeldt-Jakob Disease in a U.K. Citizen Who Had Temporarily Resided in Texas, 2001-2005

The Centers for Disease Control and Prevention (CDC) has been notified by the U.K. National Creutzfeldt-Jakob Disease (CJD) Surveillance Unit in Edinburgh, Scotland, about a probable variant CJD diagnosis in a 30-year-old man who resided in Texas during 2001-2005. The patient had onset of symptoms in early 2005 while in Texas. He then returned to the United Kingdom, where his illness progressed, and a diagnosis of variant CJD was made. The patient is currently receiving medical care in the United Kingdom.

The variant CJD diagnosis was based on typical clinical manifestations of the disease and demonstration of the characteristic “pulvinar sign” on magnetic resonance imaging of the brain. No biopsy tissues are available for pathologic confirmation of the diagnosis. While living in the United States, the patient had no history of hospitalization, of having invasive medical procedures, or of donation or receipt of blood and blood products.

The patient almost certainly acquired the disease in the United Kingdom. He was born in the United Kingdom and lived there throughout the defined period of risk (1980-1996) for human exposure to the agent of bovine spongiform encephalopathy (BSE, commonly known as “mad cow” disease). His stay in the United States was too brief relative to what is known about the incubation period for variant CJD. For additional information about the incubation period for variant CJD, see Belay ED, Sejvar JJ, Shieh WJ, et al. “Variant Creutzfeldt-Jakob Disease Death, United States,” Emerg Infect Dis 2005; available at http://www.cdc.gov/ncidod/EID/vol11no09/05-0371.htm.

By convention, variant CJD cases are ascribed to the country of initial symptom onset, regardless of where the exposure occurred. Since variant CJD was first reported in 1996, a total of 185 patients from 11 countries have been identified. As of November 2005, variant CJD cases have been reported from the following countries: 158 from the United Kingdom, 15 from France, 3 from Ireland, 2 from the United States (including the current case), and one each from Canada, Italy, Japan, Netherlands, Portugal, Saudi Arabia, and Spain. Similar to the two U.S. cases, one of the three cases from Ireland and the single cases from Canada and Japan were likely exposed to the BSE agent while residing in the United Kingdom. Strong scientific evidence indicates that variant CJD results from the transmission to humans of the agent that causes BSE in cattle. The BSE outbreak in cattle that was first detected in the 1980s in the United Kingdom has spread to many other European countries, and cases in cattle have been identified outside of Europe, in Canada, Israel, Japan, and the United States.

Statement on Texas Cow With Central Nervous System Symptoms On Friday, April 30 th , the Food and Drug Administration learned that a cow with central nervous system symptoms had been killed and shipped to a processor for rendering into animal protein for use in animal feed.

FDA, which is responsible for the safety of animal feed, immediately began an investigation. On Friday and throughout the weekend, FDA investigators inspected the slaughterhouse, the rendering facility, the farm where the animal came from, and the processor that initially received the cow from the slaughterhouse.

FDA's investigation showed that the animal in question had already been rendered into "meat and bone meal" (a type of protein animal feed). Over the weekend FDA was able to track down all the implicated material. That material is being held by the firm, which is cooperating fully with FDA.

Cattle with central nervous system symptoms are of particular interest because cattle with bovine spongiform encephalopathy or BSE, also known as "mad cow disease," can exhibit such symptoms. In this case, there is no way now to test for BSE. But even if the cow had BSE, FDA's animal feed rule would prohibit the feeding of its rendered protein to other ruminant animals (e.g., cows, goats, sheep, bison).

FDA is sending a letter to the firm summarizing its findings and informing the firm that FDA will not object to use of this material in swine feed only. If it is not used in swine feed, this material will be destroyed. Pigs have been shown not to be susceptible to BSE. If the firm agrees to use the material for swine feed only, FDA will track the material all the way through the supply chain from the processor to the farm to ensure that the feed is properly monitored and used only as feed for pigs.

To protect the U.S. against BSE, FDA works to keep certain mammalian protein out of animal feed for cattle and other ruminant animals. FDA established its animal feed rule in 1997 after the BSE epidemic in the U.K. showed that the disease spreads by feeding infected ruminant protein to cattle.

Under the current regulation, the material from this Texas cow is not allowed in feed for cattle or other ruminant animals. FDA's action specifying that the material go only into swine feed means also that it will not be fed to poultry.

FDA is committed to protecting the U.S. from BSE and collaborates closely with the U.S. Department of Agriculture on all BSE issues. The animal feed rule provides crucial protection against the spread of BSE, but it is only one of several such firewalls. FDA will soon be improving the animal feed rule, to make this strong system even stronger.

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http://www.fda.gov/bbs/topics/news/2004/NEW01061.html

AND THEN WE HAVE THE 'FONG SYNDROME' where mad cow tissue samples sit up on the shelf for 7+ months and NOT tested until the Honorable Phyllis Fong of the OIG does the end around Johanns to get the job done, then Johanns does the end around her on the next tissue sample from another mad cow, except this time, the tissue is in preservitive as to render all WB impossible. Johanns must be fired for his lies and deception on mad cow disease in the USA. ...TSS

> Date: Fri, 2 Mar 2001 23:27:10 +0000 (GMT)> From:> Subject: confidential> To: "Terry S. Singeltary Sr.">>> Okay, you need to know. You don't need to pass it on> as nothing will come of it and there is not a damned> thing anyone can do about it. Don't even hint at it> as it will be denied and laughed at..........> USDA is gonna do as little as possible until there is> actually a human case in the USA of the> nvcjd........if you want to move this thing along and> shake the earth....then we gotta get the victims> families to make sure whoever is doing the autopsy is> credible, trustworthy, and a saint with the courage of> Joan of Arc........I am not kidding!!!!> so, unless we get a human death from EXACTLY the same> form with EXACTLY the same histopath lesions as seen> in the UK nvcjd........forget any action........it is> ALL gonna be sporadic!!!>> And, if there is a case.......there is gonna be every> effort to link it to international travel,> international food, etc. etc. etc. etc. etc. They> will go so far as to find out if a sex partner had> ever traveled to the UK/europe, etc. etc. ....> It is gonna be a long, lonely, dangerous twisted> journey to the truth. They have all the cards, all> the money, and are willing to threaten and carry out> those threats....and this may be their biggest> downfall. ...end...TSS>>> Lancet Neurology 2005; 4:805-814>> DOI:10.1016/S1474-4422(05)70225-8>> Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob> disease>> Magdalini Polymenidou a, Katharina Stoeck a, Markus Glatzel a b, MartinVey> c, Anne Bellon c and Adriano Aguzzi a>> Summary> Background> The molecular typing of sporadic Creutzfeldt-Jakob disease (CJD) is basedon> the size and glycoform ratio of protease-resistant prion protein (PrPSc),> and on PRNP haplotype. On digestion with proteinase K, type 1 and type 2> PrPSc display unglycosylated core fragments of 21 kDa and 19 kDa,resulting> from cleavage around amino acids 82 and 97, respectively.>> Methods> We generated anti-PrP monoclonal antibodies to epitopes immediately> preceding the differential proteinase K cleavage sites. These antibodies,> which were designated POM2 and POM12, recognise type 1, but not type 2,> PrPSc.>> Findings> We studied 114 brain samples from 70 patients with sporadic CJD and three> patients with variant CJD. Every patient classified as CJD type 2, and all> variant CJD patients, showed POM2/POM12 reactivity in the cerebellum and> other PrPSc-rich brain areas, with a typical PrPSc type 1 migrationpattern.>> Interpretation> The regular coexistence of multiple PrPSc types in patients with CJD casts> doubts on the validity of electrophoretic PrPSc mobilities as surrogatesfor> prion strains, and questions the rational basis of current CJD> classifications.>> Affiliations>> a Institute of Neuropathology, University Hospital Zurich, Switzerland> b Present address: Institute of Neuropathology, University Medical Center> Hamburg-Eppendorf, Hamburg, Germany> c ZLB Behring, Marburg, Germany>> Correspondence to: Dr Adriano Aguzzi, Institute of Neuropathology,> University Hospital of Zürich, Schmelzbergstrasse 12, CH-8091 Zürich,> Switzerland>>>>http://www.thelancet.com/journals/laneur/article/PIIS1474442205702258/abstra> ct>>>> Received: Date: Fri, 12 Jan 2001 03:34:26 +0000 (GMT)> From:> Subject: confidential> To: flounder@wt.net>> Sorry did not get back to you. (Ran out of time!!)>> Of interest...don't repeat. On Jan 9, was somewhere> and not able to tie into conference call. Was around> an official who should have been on conference> call..another person with me also remembered it and we> both inquired as to how the call went. Was told (to> both of us) that the call had been cancelled!! (Told> us several times that the call was cancelled and they> did not know why!!!) I will try to find out why this> person said that...maybe they got off the call or they> were told to tell everyone that the call was> cancelled.> You need to POST your interaction with the conference> call on a web site....let me know when you do...I will> present this fact to the official who may have lied.> See what they have to say...but need to have the> information POSTED to a web site...send me the address> and make sure you don't mind that I give it to this> very questionable person....There must be a reason for> the lying....??? Surely people who are really> interested will found out what went on? There are> quite a few people who listened in and declined to> identify or acknowledge that they listened in...why> the big secrecy or this person may have been told to> do this. Need to know!!! Something dirty is going> on...some sort of treachery seems to be in the> works...> Not a good situation for me right now...wish I could> tell you more as to what is going on...but too> dangerous right now...got to sort it out.> Later in January hoping to meet with a trusted> individual...seems as we think alike...I think there> are allies out there, but we are just at the grass> roots timeframe. (I do think that many of us are in> angst as things seem to proceed along the route that> HIV took...I think more will be counted this time> around....still, it is important to have> "Insiders"...the world has been changed before and it> can be again....)> Another piece of information for you only: Ray> Bradley has been lying throug his teeth for years> (UK). I would not believe one word of what he says.> He has been well paid and taken care of for his SS> role in the BSE nightmare. He will say what he is> supposed to say until his dying day.........don't put> a single ounce of trust in anything he has related or> reported. He will pay the piper for all the misery he> has caused in this life...at the least in the next life....>> snip...>> Date: Sun, 21 Jan 2001 23:50:31 +0000 (GMT)> From:> Subject: stuff> To: "Terry S. Singeltary Sr." >> Confidential:> Budget: let me know what you find out and the> breakdown. There may be some stuff stuffed into it> which is not legit... They may figure some salaries> and such...the real gist of the matter is the shocking> amount of $ that is actually used to "ferret" out the> disease and the $ that are used to P.R. the whole> affair and give appearance of being concerned and> involved...again it was said years ago and it should> be taken seriously....BSE will NEVER be found in the> US!> As for the BSE conference call...I think you did a> great service to freedom of information and making> some people feign integrity...I find it scary to see> that most of the "experts" are employed by the federal> government or are supported on the "teat" of federal> funds. A scary picture!> I hope there is a confidential panel organized by the> new government to really investigate this thing. I> am sure you have seen the whitewash of the Gulf War> Syndrome that was released last Dec. There was a> senate investigation which actually showed the true> extent of the coverup...over 100,000 medical records> missing!!! but it all died down...> There is someone who is rather innovative and free> thinking...a Dr.>> snip...>> What a mess! Seems like the time is ripe for everyone> to cover their ass and start screaming...not me, not> me, not my job, etc. etc. We gotta get some big scary> folks more involved in this mess. I am meeting with> someone end of week on my own time...my cover is an> interview in the big town...Since I am always job> hunting! Ha!> You need to watch your back........but keep picking at> them.......like a buzzard to the bone...you just may> get to the truth!!!> (You probably have more support than you know. Too> many people are afraid to show you or let anyone else> know. I have heard a few things myself...you ask the> questions that everyone else is too afraid to ask.)>> snip...>>> Subject: BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001> Date: Tue, 9 Jan 2001 16:49:00 -0800> From: "Terry S. Singeltary Sr." > Reply-To: Bovine Spongiform Encephalopathy > To: BSE-L@uni-karlsruhe.de>> ######### Bovine Spongiform Encephalopathy > #########>> Greetings List Members,>> I was lucky enough to sit in on this BSE conference> call today and even managed to ask a question.> that is when the trouble started.>> I submitted a version of my notes to> Sandra Blakeslee of the New York Times,> whom seemed very upset, and rightly> so.>> "They tell me it is a closed meeting and> they will release whatever information> they deem fit. Rather infuriating.">> and i would have been doing just fine,> until i asked my question. i was surprised> my time to ask a question so quick.>> (understand, these are taken from my notes for now.> the spelling of names and such could be off.)>> [host Richard Barns]> and now a question from Terry S. Singeltary of> CJD Watch.>> [TSS]> yes, thank you,> U.S. cattle, what kind of guarantee can you> give for serum or tissue donor herds?>> [no answer, you could hear in the back ground,> mumbling and 'we can't. have him ask the question> again.]>> [host Richard]> could you repeat the question?>> [TSS]> U.S. cattle, what kind of guarantee can you> give for serum or tissue donor herds?>> [not sure whom ask this]> what group are you with?>> [TSS]> CJD Watch, my Mom died from hvCJD and we are> tracking CJD world-wide.>> [not sure who is speaking]> could you please disconnect Mr. Singeltary>> [TSS]> you are not going to answer my question?>> [not sure whom speaking]> NO>> from this point, i was still connected, got to listen> and tape the whole conference. at one point someone> came on, a woman, and ask again;>> [unknown woman]> what group are you with?>> [TSS]> CJD Watch and my Mom died from hvCJD> we are trying to tract down CJD and other> human TSE's world wide. i was invited to> sit in on this from someone inside the USDA/APHIS> and that is why i am here. do you intend on banning> me from this conference now?>> at this point the conference was turned back up,> and i got to finish listening. They never answered> or even addressed my one question, or even addressed> the issue. BUT, i will try and give you a run-down> for now, of the conference.>> IF i were another Country, I would take heed to my> notes, BUT PLEASE do not depend on them. ask for> transcript from;>> RBARNS@ORA.FDA.GOV> 301-827-6906>> he would be glad to give you one ;-)>> Rockville Maryland,> Richard Barns Host>> BSE issues in the U.S.,> How they were labelling ruminant feed?> Revising issues.>> The conference opened up with the explaining of> the U.K. BSE epidemic winding down with about 30> cases a week.>>> snip...full text 50 state bse conference call ;>> http://www.vegancowboy.org/TSS-part1of8.htm>> http://www.vegancowboy.org/TSS-Shortcut-parts1to8.htm>>> TSS>>> ----- Original Message -----> From: "Terry S. Singeltary Sr." > To: > Sent: Monday, November 21, 2005 7:58 AM> Subject: Brit-born Texan diagnosed with vCJD>>> ##################### Bovine Spongiform Encephalopathy> #####################>>> TSS> Brit-born Texan diagnosed with vCJD> Mon Nov 21, 2005 07:55> 68.238.103.140>>> Brit-born Texan diagnosed with vCJD by Pete Hisey on 11/21/2005 for> Meatingplace.com A 30-year-old British male living in Houston is believedto> be the second victim of variant Creutzfeldt-Jakob disease, the human formof> bovine spongiform encephalopathy, in the United States.>> The victim moved to Houston in 2001 to work for Shell Oil after spending> most of his life in Great Britain. The Centers for Disease Control and> Prevention planned to issue a report with more detail over the weekend.>> The victim apparently alarmed relatives during phone calls home this year> with changes in his personality. An MRI and examination resulted in the> diagnosis of probable vCJD. According to his family, the victim has no> history of risk factors such as surgery, blood transfusions or IV drug use> during his time in Texas.>> While it appears that he was exposed to the disease while living in the> United Kingdom, he will be considered a U.S. victim for purposes of> reporting the disease.>>>> http://www.meatingplace.com/MembersOnly/webNews/details.aspx?item=15166>>> TSS>> #################### https://lists.aegee.org/bse-l.html> ####################